AB0581 TRACHEAL STENOSIS; AN IMPORTANT INVOLVEMENT IN GRANULOMATOSIS WITH POLYANGIITIS
Authors: O. Ozdemir Isik, F. Tuncer, O. Sadioglu Cagdas, A. Yazici, A. Cefle
Journal: Annals of the Rheumatic Diseases (2022)

Background Granulomatosis with polyangiitis (GPA) is a granulomatous systemic vasculitis of unknown etiology that can affect many organs. Approximately 90% of patients with GPA have upper respiratory tract involvement, including the nasal cavity, sinuses, ear and trachea. Subglottic stenosis (SGS) may develop in some patients due to tracheal involvement. Objectives It is aimed to evaluate the general characteristics, treatments and disease prognosis of our GPA cases with tracheal stenosis as it is a significant cause of morbidity and mortality. Methods The data of 48 patients diagnosed with GPA between 2000-2021 were analyzed retrospectively and the data of 6 patients with tracheal stenosis (TS) were evaluated. Results TS was present in 13% of the patients. All patients with TS were female. The mean age of the patients with TS was 46.5±6.5 years, the mean age of disease onset was 35.5 ±13.4 years, and the mean disease duration was 10.8±10.2 years. Constitutional symptoms were in 17% of patients, and 17% had mastoiditis, sinusitis, otitis, bloody nasal discharge and renal involvement. None of the patients had hearing loss, skin, eye and neurological involvement. 83% of patients had limited disease. While all patients had SGS, one patient also had glottic and bronchial stenosis. The most common symptoms in patients were hoarseness and shortness of breath. All patients received systemic treatment according to their organ involvement, local treatment (dilatation, steroid injection) was also applied to two patients. All patients had anti-neutrophil cytoplasmic antibody (ANCA) positivity. When patients with and without tracheal stenosis were compared, there was a difference in terms of gender, lung and kidney involvement, presence of anemia, increased erythrocyte sedimentation rate, pANCA, cANCA and PR3-ANCA positivity, and myalgia. In addition, our patients with tracheal stenosis had a younger age of onset and a longer delay in diagnosis. Conclusion The incidence of SGS in large series has been reported as 8-23% of GPA patients. 1,2 Although the number of cases in our study was small, this rate was 13%. Symptoms range from cough and shortness of breath to progressively life-threatening severe stridor. Early diagnosis and treatment are important. For this reason, TS is strongly emphasized. References [1]Girard C, Charles P, Terrier B, Bussonne G, Cohen P, Pagnoux C, et al. Tracheobronchial stenoses in granulomatosis with polyangiitis (Wegener’s). Medicine. 2015;34:1-6. [2]Costantino CL, Niles JL, Wright CD, Mathisen DJ, Muniappan A. Subglottic stenosis in granulomatosis with polyangiitis: therole of laryngotracheal resection. Ann Thorac Surg 2018;105:249-53. Table 1. Comparison of data from GPA patients with and without tracheal stenosis N(%) Tracheal Stenosis +GPA(n=6) Tracheal Stenosis - GPA (n=42) P Gender Female 6 (100) 21(51) 0.031 Male 0 20(49) Mean Age 46.5±6.5 55.6±13.2 0.095 Symptom Onset Age 35.5±13.4 49.3±14.5 0.041 Delay in Diagnosis (months) 44.2±6.5 9.98±14.8 0.023 Myalgia 2(33) 33(81) 0.030 Fever 1(17) 21(51) 0.194 High ESR 1(17) 31(78) 0.007 High CRP 5(50) 32(80) 0.138 Anemia 1(17) 28(70) 0.020 Upper Respiratory Involvement 6(100) 24(59) 0.074 Lung Involvement 3(50) 36(90) 0.037 GIS involvement 1(17) 6(15) 1 Eye Involvement - 11(27) NA Skin Involvement - 12(29) NA Urogenital Involvement - 1(2) NA Neurological Involvement - 13(32) NA Renal Involvement 1(17) 29(71) 0.018 ANCA positivity 6(100) 38(93) 1 MPO-ANCA 2(33) 4(10) 0.162 PR3-ANCA 1(17) 33(81) 0.004 p-ANCA 5(83) 6(15) 0.000 c-ANCA 1(17) 31(78) 0.001 Disclosure of Interests None declared